Evaluation of Optical Coherence Tomography Angiography Findings in Pars Planitis and Multiple Sclerosis Associated Intermediate Uveitis in Remission.

PURPOSE: To evaluate the microvasculature during remission in patients with pars planitis (PP-IU) and multiple sclerosis-associated intermediate uveitis (MS-IU) using optical coherence tomography angiography (OCT-A). METHODS: Single-center, descriptive, case-control study was conducted. Adult patients (≥16 years) with IU in remission (PP-IU and MS-IU) and healthy age-sex matched healthy controls (HC) were enrolled to the study. Demographic/clinical features, best-corrected visual acuity (BCVA), ocular findings, neurological symptoms and preferred treatments were recorded. The presence of cystoid macular edema (CME) during follow-up was recorded. All IU patients in remission and HC subjects were scanned with OCT-A. Foveal avascular zone (FAZ) areas of superficial and deep capillary plexus (SCP/DCP) and vascular densities of SCP, DCP and choriocapillaris were obtained from OCT-A and compared between the groups. RESULTS: Sixty-nine eyes of 37 IU patients in remission and 20 HC were included (44 eyes/23 patients in PP-IU, 25/14 in MS-IU, 40/20 in HC). No statistically significant differences were observed in terms of demographic or clinical characteristics of the patients. The vascular density in the SCP was significantly reduced in the PP-IU and MS-IU groups compared to the HC group (p < .05). Nevertheless, there were no significant changes in any of the OCTA parameters between the IU groups. Uveitis duration was found to be correlated with enlargement of the FAZ area in PP-IU (p = .039). CONCLUSION: OCTA may not be useful in differentiating between PP-IU and MS-IU. Nevertheless, the primary implication in SCP potentially elucidates the pathogenesis of these two subtypes of IU, which are characterized by a shared pathogenesis. The monitoring of the FAZ area in the PP-IU group is valuable in terms of chronicity.

Pediatric Pars Planitis: A Review.

PURPOSE: To provide an overview of pediatric pars planitis. METHODS: Narrative literature review. RESULTS: Pars planitis refers to the idiopathic subset of intermediate uveitis in which there is vitritis along with snowball or snowbank formation occurring in the absence of an associated infection or systemic disease. It is thought to be a T-cell mediated disease with a genetic predisposition. Pars planitis accounts for 5-26.7% of pediatric uveitis cases. Presentation is commonly bilateral but asymmetric, often with insidious onset of floaters and blurred vision. Although pars planitis is known to be a benign form of uveitis in most cases, severe complications secondary to chronic inflammation may arise, with cystoid macular edema being the most common cause of visual morbidity. Mild vitritis in the absence of symptoms, vision loss, or macular edema may be observed. Patients with severe vitritis and/or associated vision-threatening complications require prompt aggressive treatment. A stepladder approach including corticosteroids, immunosuppressive agents, anti­tumor necrosis factor­alpha and pars plana vitrectomy and/or laser photocoagulation is the most commonly used method for treatment of pars planitis. CONCLUSION: Timely diagnosis and adequate treatment of pediatric pars planitis and associated complications are crucial in order to improve visual outcomes.

Clinical characteristics of intermediate uveitis in adults according to criteria of the SUN working group.

PURPOSE: To analyse the clinical characteristics of adult patients with pars planitis (PP-IU), non-pars planitis (NPP-IU) and multiple sclerosis-associated intermediate uveitis (MS-IU) and distinguish between groups. METHODS: Seventy-three adult patients with intermediate uveitis (IU) reviewed retrospectively and divided as PP-IU, NPP-IU and MS-IU according to 'The standardization of uveitis nomenclature working group classification criteria.' Demographic and clinical characteristics, OCT and fluorescein angiography (FA) findings, complications and treatments were recorded. RESULTS: A total of 134 eyes of 73 patients were included, and 42 of the patients were classified as PP-IU, 12 as NPP-IU, and 19 as MS-IU. If a patient presenting with blurred vision, or tent-shaped vitreous band/snowballs/snowbank on examination, or vascular leakage on FA and accompanying neurological symptoms, the frequency of demyelinating plaque detection on cranial MRI and the risk of MS-IU increased. Mean BCVA was increased from 0.22 ± 0.30 logMAR to 0.19 ± 0.31 logMAR (p = 0.021). Gender, initial BCVA, snowbank formation, disc oedema and periphlebitis on examination, and disc leakage/occlusion on FA were found predictive of decreased BCVA at final visit (p < 0.05). CONCLUSIONS: The clinical features of these three groups are similar, some features that can guide the differential diagnosis. It may be recommended to periodically evaluate "suspicious" patients with MRI for MS.

A Population-Based Analysis of Long-Term Costs and Adverse Events after Pneumatic Retinopexy and Pars Plana Vitrectomy.

PURPOSE: To comprehensively examine the cost effectiveness, reattachment rate, and complications of pneumatic retinopexy (PnR) compared with pars plana vitrectomy (PPV) for rhegmatogenous retinal detachment (RRD) within a universal health care system. DESIGN: Population-based, multicenter, consecutive, retrospective longitudinal cohort analysis. SUBJECTS: We identified consecutive adults aged ≥ 50 years requiring surgery for primary RRD over a 20-year interval between April 1, 2002, and March 31, 2022. Initial surgery was considered the index date for analyses. INTERVENTION: Pneumatic retinopexy was compared with PPV in all analyses. MAIN OUTCOME MEASURES: The primary analysis investigated the mean annualized health care costs comparing PnR to PPV over the 2 years after initial surgery. Secondary analyses examined the primary reattachment rate and complications. RESULTS: In total, 25 665 eligible patients were identified, with 8794 undergoing PnR and 16 871 undergoing PPV. The mean patient age was 65 years and 39% were women. The mean annualized cost after PnR was $8924 and $11 937 after PPV (mean difference, $3013; 95% confidence interval, $2533-$3493; P < 0.001). The primary reattachment rate at 90 days after PnR was 83% and after PPV was 93% (P < 0.001). The risk of cataract or glaucoma surgery was lower after PnR, and the frequency of ophthalmology clinic visits, intravitreal injections, and anxiety was higher after PnR. Hospitalizations and long-term disability were less frequent after PnR. CONCLUSIONS: Pneumatic retinopexy, when compared with PPV, was associated with lower long-term health care costs. Pneumatic retinopexy appeared to be effective, safe, and inexpensive, thus offering a viable option for improving access to RRD repair in appropriately selected cases. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

A New Technique of Pars Plana Vitrectomy With Microinterventional Nucleus Disassembly to Reduce the Energy of Fragmatome Lensectomy for Posteriorly Dislocated Crystalline Lens Removal.

PURPOSE: To describe a novel technique of lens disassembly in posteriorly dislocated crystalline lens removal. METHODS: A microinterventional microfilament loop device was introduced through the sclerotomy created for the fragmatome to cleave the lens into smaller, manageable pieces. RESULTS: Five cases were performed with this technique with improved visual acuity and no complications. CONCLUSION: The use of microinterventional nucleus disassembly during pars plana vitrectomy for retained lens material is a novel approach with potential advantages including decreased operating time and reduced complications secondary to excessive production of ultrasound energy.

Anti-tumor necrosis factor-alpha treatment in pediatric patients with pars planitis: a single-center experience from Turkey.

AIM: To evaluate the effectiveness and safety of anti-tumor necrosis factor-alpha (anti-TNF-alpha) treatment (Adalimumab [ADA]) combined with immunomodulatory agents (IMAs) in the treatment of pars planitis (PP). METHODS: The patients with PP who were treated with anti-TNF-alpha agents for at least six months were qualified for the chart review. The outcome parameters were the steroid-free remission state, the best-corrected visual acuity (BCVA) and the central macular thickness (CMT) of the patients at the last visit. RESULTS: After a mean total follow-up time of 15.5 ± 5.8 months (8-24 months), all the cases were in steroid-free remission at the last visit. The mean BCVA increased, and the mean CMT decreased significantly at the last visit (p < 0.001, p < 0.001, respectively). CONCLUSION: ADA combined with IMAs offers effective and safe treatment modalities in the control of chronic intraocular inflammation in PP cases.

Intermediate Uveitis: A Review.

PURPOSE: This review aims to provide an update on the clinical presentation, etiologies, complications, and treatment options in intermediate uveitis (IU). METHODS: Narrative literature review. RESULTS: IU affects all age groups with no clear gender predominance and has varied etiologies including systemic illnesses and infectious diseases, or pars planitis. In some instances, IU may be the sole presentation of an underlying associated condition or disease. Management of IU and its complications include administration of corticosteroids, antimetabolites, T-cell inhibitors, and/or biologics, along with surgical interventions, with varying degrees of effectiveness across literature. In particular, increasing evidence of the safety and efficacy of immunomodulatory agents and biologics has seen greater adoption of these therapies in clinical practice. CONCLUSIONS: IU is an anatomical description of uveitis, involving intraocular inflammation of the vitreous, peripheral retinal vasculature, and pars plana. Various treatment options for intermediate uveitis are currently used in practice.

Pediatric Uveitis in Turkey: The National Registry Report II.

PURPOSE: To report demographic and clinical profiles of children with uveitis in theTurkishpopulation. METHODS: The data of the pediatric uveitis cases in the nation wide uveitis database were analyzed. RESULTS: The study included 697 eyes of 442 patients with a meanage of 10.8 ± 3.8 years. There were 333 patients (75.3%) with non-infectious uveitis and 69 patients (15.6%) with infectious uveitis. Pars planitis (20.1%) was leading clinical form followed by idiopathic cases with uveitis other than pars planitis (18.8%), juvenile idiopathic arthritis(JIA) related uveitis (12.4%), Behçet uveitis (9.3%) and toxoplasma retinochoroiditis (7.9%). Ocular involvement was unilateral in 187 patients (42.3%) and bilateral in 255 patients (57.7%). The most common anatomiclocation of uveitis was anterior uveitis (39.1%), followed by intermediate uveitis (29.4%), panuveitis (16.1%) and posterior uveitis (15.4%). CONCLUSION: The most common systemic association was JIA in the younger children and Behçet disease in the older children.

Retinoblastoma in an older Hispanic child masquerading as pars planitis: A case report.

INTRODUCTION: To report a case of a Hispanic girl with late-onset Retinoblastoma (Rb) who was misdiagnosed as a pars planitis prior to referral. Nearly 95% of all Rb cases are detected before age 5, and this patient was 8 years-old. METHODS: Case report of a late-onset Retinoblastoma with anterior chamber (AC) involvement plus the presence of an Ahmed valve. The patient had a history of a couple of months of topical therapy comprising medication for glaucoma, systemic steroids, and a filtration surgery (Ahmed valve), after that a biopsy was performed prior to referral. Upon arrival at our clinic, we performed an examination under anesthesia (EUA) and a B-scan ultrasound (US). RESULTS: Unilateral Retinoblastoma with an Ahmed valve in an AC filled with Rb seeds was diagnosed with the EUA and US in the left eye. An orbital exenteration with map biopsies of the left orbital cavity was performed with confirmation by histopathology of a poorly differentiated endophytic retinoblastoma with Bruch's membrane invasion. Follow-up sessions were then arranged as well as subsequent systemic chemotherapy cycles. CONCLUSION: Given the rare incidence of retinoblastoma in children older than 5 years old, it can be easily mistaken for other differential diagnoses and treated with filtration surgeries that could put the patient's life at risk. In this report, late-onset Rb diagnosis is highlighted as a differential diagnosis in children and adults with atypical uveitis, which required a multidisciplinary approach.

Clinical characteristics and treatment of pars planitis: an adalimumab experience.

PURPOSE: This study aims to investigate the clinical and demographic characteristics, treatment outcomes and complications of patients with pars planitis. METHODS: This retrospective study included patients diagnosed with pars planitis between 1998 and 2019 and followed for at least 6 months. Demographics, best-corrected visual acuity (BCVA), anterior segment and fundus examination findings, intraocular pressure (IOP) values at baseline and final examination, treatments used during the follow-up, surgeries and complications were noted from medical records of the patients. The percentage of patients given adalimumab (ADA), the reasons for treatment switch and response to ADA were investigated. RESULTS: One hundred fifteen eyes of 59 patients were included in the study. Forty-seven percent of patients were female. The median age of the patients was 10 (4-44) years. The median follow-up time was 33 (6-252) months. The median BCVA at admission was 0.20 (0.00-2.00) logMAR. The most common complications were cystoid macular oedema, cataract, epiretinal membrane and inferior peripheral retinoschisis. Prophylactic laser photocoagulation for peripheral retinoschisis was the most common surgical intervention, followed by cataract surgery and pars plana vitrectomy. Approximately 80% of patients received immunosuppressive and corticosteroid therapy for initial treatment. ADA was initiated in 23 patients (38.9%) due to refractory uveitis and adverse effects to the corticosteroid and helped control intraocular inflammation and decrease the use of systemic steroids/immunosuppressives in 22 of 23 (95%) of patients who received ADA. The median BCVA at final examination increased to 0.00 (0.00-2.00) logMAR. CONCLUSIONS: Pars planitis is a chronic, progressive and insidious disease with several ocular complications and requires early and aggressive treatment. ADA appeared to be effective especially in patients' refractory to conventional treatment.

Classification Criteria For Pars Planitis.

PURPOSE: To determine classification criteria for pars planitis. DESIGN: Machine learning of cases with pars planitis and 4 other intermediate uveitides. METHODS: Cases of intermediate uveitides were collected in an informatics-designed preliminary database, and a final database was constructed of cases achieving supermajority agreement on the diagnosis, using formal consensus techniques. Cases were split into a training set and a validation set. Machine learning using multinomial logistic regression was used on the training set to determine a parsimonious set of criteria that minimized the misclassification rate among the intermediate uveitides. The resulting criteria were evaluated on the validation set. RESULTS: Five hundred eighty-nine cases of intermediate uveitides, including 226 cases of pars planitis, were evaluated by machine learning. The overall accuracy for intermediate uveitides was 99.8% in the training set and 99.3% in the validation set (95% confidence interval 96.1, 99.9). Key criteria for pars planitis included unilateral or bilateral intermediate uveitis with either 1) snowballs in the vitreous or 2) snowbanks on the pars plana. Key exclusions included: 1) multiple sclerosis, 2) sarcoidosis, and 3) syphilis. The misclassification rates for pars planitis were 0% in the training set and 1.7% in the validation set, respectively. CONCLUSIONS: The criteria for pars planitis had a low misclassification rate and appeared to perform sufficiently well for use in clinical and translational research.

Classification Criteria for Intermediate Uveitis, Non-Pars Planitis Type.

PURPOSE: To determine classification criteria for intermediate uveitis, non-pars planitis type (IU-NPP, also known as undifferentiated intermediate uveitis). DESIGN: Machine learning of cases with IU-NPP and 4 other intermediate uveitides. METHODS: Cases of intermediate uveitides were collected in an informatics-designed preliminary database, and a final database was constructed of cases achieving supermajority agreement on the diagnosis, using formal consensus techniques. Cases were split into a training set and a validation set. Machine learning using multinomial logistic regression was used on the training set to determine a parsimonious set of criteria that minimized the misclassification rate among the intermediate uveitides. The resulting criteria were evaluated on the validation set. RESULTS: Five hundred eighty-nine of cases of intermediate uveitides, including 114 cases of IU-NPP, were evaluated by machine learning. The overall accuracy for intermediate uveitides was 99.8% in the training set and 99.3% in the validation set (95% confidence interval 96.1, 99.9). Key criteria for IU-NPP included unilateral or bilateral intermediate uveitis with neither snowballs in the vitreous humor nor snowbanks on the pars plana. Other key exclusions included multiple sclerosis, sarcoidosis, and syphilis. The misclassification rates for IU-NPP were 0% in the training set and 0% in the validation set. CONCLUSIONS: The criteria for IU-NPP had a low misclassification rate and seemed to perform well enough for use in clinical and translational research.

Clinical Course and Treatment Paradigms for JIA-related Uveitis and Pars Planitis Uveitis Using Precise Ocular Investigational Methods.

PURPOSE: The aim of this study was to report on the disease course and management modalities in patients with juvenile idiopathic arthritis-related uveitis (JIA uveitis) or intermediate uveitis of the pars planitis type (PP) who were followed up using precise ocular investigational techniques. METHODS: This is a retrospective single-centre study. All charts of patients seen in our centre over 15 years (2005 - 2019) with a diagnosis of JIA uveitis or PP were retrieved and analysed for clinical course, severity of inflammation, type of management, and the role played by precise investigational techniques (laser flare photometry [LFP], optical coherence tomography [OCT] and fluorescein angiography [FA]) in therapeutic decisions. RESULTS: 26 out of a total of 64 patients with sufficient data could be included in the study. Mean age was 11.2 years (SD ± 5.4). 13/26 (50%) patients presented with JIA uveitis and 13/26 (50%) with PP. PP patients had a more benign course (mean LFP values at presentation 9.9 ± 3.2 ph/ms), and at the end of follow-up, only 2/13 PP patients (15%) still needed systemic treatment. In contrast, JIA uveitis as a whole was more severe, although benign forms were present (LFP values at presentation 105.9 ± 19.5 ph/ms), and at the end of follow-up, 5/13 patients (38%) still needed systemic treatment. Complications were also more severe and frequent in JIA uveitis patients. In 6/26 patients (24%, 3 JIA and 3 PP patients), the precise monitoring methods allowed unjustified systemic treatment to be discontinued, and to avoid such a treatment, it was recommended that it should be replaced by topical treatment or observation. CONCLUSION: Nowadays, new investigational techniques have made precise follow-up of uveitis possible. We determined the precise inflammatory pattern of JIA uveitis and PP, which is crucial information to determine the therapeutic intervention. As these two entities are common in young and paediatric patients, such precise monitoring is essential to determine adequate treatment paradigms and avoid unnecessary systemic treatment, especially corticosteroids. When the ophthalmic status requires it, multidisciplinary collaboration between the ophthalmologist, the paediatrician and the rheumatologist may be needed to offer optimal management to the patient. In cases of purely ocular involvement, it is the ophthalmologist who should determine the management.

Spectral domain optical coherence tomography findings of patients with pars planitis and risk factors affecting visual acuity.

PURPOSE: To evaluate spectral domain optical coherence tomography (SD-OCT) findings of 42 eyes with pars planitis and to identify risk factors affecting visual acuity. METHODS: Medical records and SD-OCT findings were retrospectively reviewed. RESULTS: Mean best-corrected visual acuity (BCVA) was 0.248 ± 0.3 on the logMAR scale at baseline. SD-OCT findings included epiretinal membrane (ERM) in 16 (38.1%) eyes, loss of normal foveal contour appearance in 8 (19.0%), ellipsoid zone (EZ) damage in 5 (11.9%), external limiting membrane (ELM) damage in 3 (7.1%), disruption of retinal pigment epithelium (RPE) in 2 (4.8%), and macular atrophy in 1 (2.4%). There was macular edema in 10 (23.8%) eyes [cystoid macular edema (CME) in 8 (19.0%), diffuse macular edema (DME) in 6 (14.3%), and serous retinal detachment in 2 (4.8%)]. The mean central macular thickness (CMT) was 272.1 ± 319.5 µm. Patients were followed up for a mean of 50.6 ± 36.7 months. Mean BCVA was 0.210 ± 0.3 at the final evaluation. SD-OCT findings included ERM in 28 (66.7%) eyes, EZ damage in 6 (14.3%), ELM damage in 3 (7.1%), disruption of RPE in 4 (9.5%), loss of normal foveal contour appearance in 12 (28.6%), and macular atrophy in 2 (4.8%). There was CME and/or DME in 6 (14.3%) eyes. The mean CMT was 238 ± 220.9 µm and was significantly lower than the baseline (p < 0.001). According to multivariate linear regression analysis, the presence of DME, and loss of normal foveal contour appearance at baseline were the independent factors associated with BCVA at the final examination (B = 0.726, p < 0.001; B = 0.766, p < 0.001, respectively). CONCLUSIONS: DME and loss of normal foveal contour appearance were more likely to have adverse effects on visual acuity.

Imbalance in PB IL-17-Secreting and Regulatory Cells in Pars Planitis Is Associated with Dysregulation of IFN-γ-Secreting Cells, Especially in Patients with Clinical Complications.

RESULTS: In patients, an increase in the population of Th17-secreting cells negatively correlated with the abundance of both IFN-γ-producing and T regulatory as well as suppressor cells, regarding all the phenotypes studied. Although a strong dependence of the PB Th1 cell compartment on the duration of the disease was observed, it was limited to the subgroup of patients with macular edema only. The frequency of B regulatory cells was unchanged compared to controls. CONCLUSIONS: In pars planitis, the alterations in lymphocyte cell distribution affect primarily the T cell repertoire. The imbalance in PB Th1/Th17/Treg cells creates proinflammatory conditions, strengthening the suggestion that the immune background may play a role in pars planitis pathogenesis. Also, circulating Th1 level may be of potential clinical relevance in terms of prediction of a more severe course of the disease.

La esclerosis múltiple en oftalmología: más allá de la neuritis óptica / Multiple sclerosis in ophthalmology: beyond optic neuritis

Las alteraciones del sistema visual son uno de los principales rasgos de la esclerosis múltiple (EM) y conllevan un gran impacto en la calidad de vida de los pacientes. Aunque la neuritis óptica es la manifestación más frecuente, existen otros procesos oftalmológicos no relacionados con la neuritis, cuyo conocimiento es de gran utilidad en el manejo de los pacientes con EM. Se describen estas anomalías agrupadas en alteraciones de las vías aferente y eferente, así como de áreas cerebrales superiores. Adicionalmente, se describen los principales efectos oftalmológicos secundarios de los fármacos actualmente empleados en el control de la EM

Disorders of the visual system are one of the main features of multiple sclerosis (MS), and have a great impact on the quality of life of patients. Although optic neuritis is the most frequent manifestation, there are other ophthalmological processes not related to neuritis, a knowledge of which is very useful in the management of patients with MS. These abnormalities are described, grouped into impairments of the afferent pathway, efferent pathway, or upper cerebral areas. Additionally, the main ophthalmological side effects of the drugs currently used in the control of MS are described

Implications of pars planitis-associated cystoid macular edema on visual outcome and management in children.

PURPOSE: Pars planitis is a commonly observed type of pediatric uveitis. The aim of this study was to evaluate the implications of pars planitis-associated cystoid macular edema (CME) on visual outcome and treatment modalities. METHODS: A retrospective review of medical records in a single center with academic practice. RESULTS: Included were 33 children (mean age 8 years, 58 eyes). Eighteen eyes developed CME (31%): in 67% of them, CME was diagnosed at presentation and in 33%, it developed at a mean of 57 months after presentation. Anterior and posterior segment complications were more prevalent in eyes with CME. Papillitis was significantly associated with the development of CME (OR 12.4, 95% CI 2.3 to 65.6, p = 0.003). Patients with CME were 1.7 times more likely to be treated with systemic therapy. By the last follow-up, 50% of patients who never developed CME were without systemic therapy compared with 13% of patients who developed CME (p = 0.034). LogMAR visual acuity improvement between presentation and month 36 was 0.41 for eyes with CME compared with 0.14 for eyes that never developed CME (p = 0.009). CONCLUSION: Pars planitis-associated CME entailed higher prevalence of ocular complications, more frequent use of immunomodulatory therapy, and a lower rate of remission.

Clinical Presentation, Management, and Long-Term Outcome of Pars Planitis, Panuveitis, and Vogt-Koyanagi-Harada Disease in Children and Adolescents.

OBJECTIVE: Chronic uveitis is a common manifestation of pediatric rheumatologic conditions and may result in irreversible blindness and long-term disability. While chronic anterior uveitis is the most commonly encountered ocular manifestation of rheumatic disease, little is known about the clinical presentation, management, and long-term outcome of more complex eye conditions such as pars planitis (PP), panuveitis (PU), and Vogt-Koyanagi-Harada disease (VKH). The present study was undertaken to comprehensively assess the long-term safety and efficacy of disease-modifying antirheumatic drugs (DMARDs) and biologics for the treatment of pediatric and adolescent patients with PP, PU, and VKH. METHODS: We retrospectively reviewed a cohort of 75 children and adolescents with idiopathic PP (n = 50), PU (n = 12), and VKH (n = 14) followed by the Pediatric Rheumatology Core at Children's Hospital Los Angeles and evaluated referral patterns, clinical presentation, treatment response, and long-term clinical outcome. RESULTS: Patients were followed for an average of 52 months. Their mean age at disease onset was 10 years. Bilateral eye involvement was seen in 87% of the patients. At first presentation to an ophthalmologist, glaucoma was noted in 21% of patients and vision loss (<20/40) in 87% of patients, while legal blindness (≤20/200 in the better-seeing eye) was diagnosed in 18 of 75 (24%) of patients (PP 22%, PU 36%, and VKH 21%). The average referral time to a pediatric rheumatologist was 13 months (range 1-96 months). Topical steroids were used in all patients, but 98% of patients required additional DMARDs, and 73% required therapy with biologics. After a mean of 52 months, 35% of patients across all disease groups had significant vision loss or were blind, and only 28% were in clinical remission without medications. The worst outcome was observed in children with PU. Regression analysis, young age at onset, delayed referral to a pediatric rheumatologist, and chronic disease were strong predictors for the risk of long-term blindness. CONCLUSION: PP, PU, and VKH involve a high risk of permanent vision loss and should be managed by a skilled rheumatologist as early and as aggressively as possible.